Selection of Operative Approach in Children with Currarino Syndrome

doi:10.21203/rs.3.rs-1950666/v1

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Selection of Operative Approach in Children with Currarino Syndrome

https://doi.org/10.21203/rs.3.rs-1950666/v1

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published 06 Jan, 2023

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Purpose: To summarize the experience of surgical treatment of children diagnosed with Currarino syndrome, with an emphasis on selection of optimal operative approach.

Methods: The clinical materials of patients diagnosed with Currarino syndrome were recorded. Special attention was paid to the operative management, particularly the different routes for operation. The type of ARM was the critical point. Follow-up was conducted for at least 3 months, and the Rintala score was used for evaluation of bowel function.

Results: The medical records of 26 patients were reviewed and listed in table one. Seven were male, and 19 were female, with a mean age of 19.38±13.80 months (ranging from 2 to 47 months). The anorectal malformation included 17 rectoperineal fistulae without an anus, four rectovestibular fistulae without an anus, two anal stenoses, one anal atresia, one rectourinary fistula without an anus, and one cloaca. The PS was taken in 23 cases. The SPS was taken in eight cases, and LPS was taken in another 15 patients. SPS group included three rectoperineal fistulae without an anus, one anal stenosis, one retraction of the rectum after an anoplasty for rectovestibular fistula without an anus, one anal atresia, one rectourinary fistula without an anus, and one cloaca. In the LPS group, there were 13 perineal fistulae without an anus，one displacement of the rectum and one retraction of the rectum respectively after an anoplasty for rectovestibular fistula without an anus. TA was used in one anal atresia with megacolon and presacral mass. AS was used in one rectovestibular fistula with a presacral mass. A lamina approach was chosen for one patient with an infective fistula secondary to a presacral mass in the spinal canal. The mean follow-up time was 39.48±26.84m, ranging from 6m to 145m. The Rintala score was16.74±2.93, ranging from 12 to 20.

Conclusion: For a rectoperineal fistula, SPS or LPS could be used to reach a complete cutback of the posterior wall of the rectum and remove the presacral mass. For a rectovestibular fistula, the AS or LPS could be chosen. For anorectal stenosis, TA, SPS, or LPS could be used to excise the stenosed bowel segment and the presacral mass. TA is not so good for the exposure of the presacral mass, so for a larger one(＞5cm) or one connecting with the spinal canal, the LPS or SPS may be the favorite choice.

Currarino syndrome

Operative

Approach

Megacolon

Currarino syndrome is a rare triad consisting of sacral dysgenesis, presacral mass, and anorectal malformation(ARM). It can present as an imperforate anus at birth, abdominal distension, constipation, obstipation from anorectal stenosis or extrinsic compression from a presacral mass, or acute or recurrent meningitis[1, 2]. Operative management of Currarino syndrome includes resectioning the presacral mass and rectifying the anorectal malformation. And treatment by anterior sagittal approach(AS), posterior sagittal approach( PS) or, transanal approach(TA) may be used with the cooperation of pediatric and neurological surgeons for optimal results[3-5]. The standard posterior sagittal approach (SPS) is the most common approach in case reports[6,7]. Also, the limited posterior sagittal approach (LPS) may be used[8]. However, there has been no study on choosing the optimal one. The reason includes that Currarino syndrome is rare, and delayed or missed diagnosis is common.

Our hospital is a tertiary hospital in Asia, and Currarino syndrome is more common than in Europe or America. This study reviewed the materials of children diagnosed with Currarino syndrome in our institute. We paid particular attention to how to choose the optimal operative approach.

From January 2008 to September 2021, the clinical materials of children diagnosed with Currarino syndrome in our department were retrospectively studied. Clinical data were extracted from the case records. The case history, clinical manifestations, imaging findings, surgical treatment, pathology, and results were summarized. The study was under the ethical standards of the ethical committee of our hospital.

Special attention was paid to the operative management, particularly the different routes for operation. The type of ARM was the critical point. Meanwhile, the size of the mass, its relation to the spinal canal, and coexisted megacolon were the other factors we should consider.

For imperforate anus with rectoperineal fistula

The child was positioned to a lithotomy position. The center of the external sphincter was mapped using an electrical stimulator, and usually an LPS incision was made. After splitting the posterior wall of the rectum, the presacral space was exposed for mass resection. Small and low presacral masses could be resected through the approach, while large and high presacral masses need an SPS incision.

For anorectal stenosis

The child was positioned to a lithotomy position. TA approach could be chosen. Then the dissection was started 1 cm above the dentate line. If there was megacolon, an endorectal pull-through should be performed. The dilated rectum was mobilized to be retracted without tension. For its adhesion to the posterior wall of the rectum, the presacral mass should be resected with mobilization of the rectum. Small and low presacral masses could be resected through a TA approach, while large and high presacral masses may need a PS approach.

For imperforate anus with rectourethral fistula

The child was placed in the prone position. The center of the external sphincter was mapped using an electrical stimulator. The SPS incision separates the muscle complex and levator muscles in the midline. Then, the rectum was located. After removing the presacral mass, the distal rectum with fistula was mobilized. The rectum was then pulled through the tunnel to the perineal wound.

For imperforate anus without fistula

The child was placed in the prone position. The blind end of the rectum was usually located at the level of the bulbar urethra and was easily reachable from the SPS approach. The rectum must be carefully separated from the urethra because the two structures share a common wall. The resection of the presacral mass was performed before this. Then the anoplasty was performed like that for the rectourethral ﬁstula.

For imperforate anus with rectovestibular fistula

The child was positioned to a lithotomy position. The center of the external sphincter was mapped using an electrical stimulator externally. The midline incision was made at the site of the proposed anal orifice and extended anteriorly (AS) for resection of the presacral mass and anoplasty.

For cloaca

First, a cystoscopy and vaginoscopy should be performed for surgical planning. The position of the rectum must be carefully checked to see whether it can be reached from a posterior sagittal incision or will require a laparotomy or laparoscopy. The repair of cloacal malformations with a less than 3 cm common channel can be performed through an SPS alone, usually using a total urogenital mobilization technique. Long common-channel cloacas were less predictable and frequently require vaginal replacement and reconstructive urological expertise. The presacral mass should be resected with mobilization of the rectum.

For megacolon

The grossly dilated rectum and sigmoid could be mobilized with laparotomy or laparoscopy. The proximal colon was retracted to the position of the anus without tension. Distal dissection was performed 1 cm above the dentate line. The anterior rectum was mobilized at the submucosal plane. The posterior rectum was mobilized at full thickness together with the presacral mass, which always had adhesion with the posterior wall of the rectum. After resectioning the dilated colon, the proximal colon was anastomosed to the anus.

Time for enterostomy

Enterostomy was suggested for patients with previous surgical history to ensure wound healing. As to a presacral mass connected with the spinal canal, enterostomy was obligatory to prevent a fatal CNS infection.

The follow-up was conducted in the outpatient department or by phone. The Rintala bowel function score was used for evaluation of bowel function[9]. The patients were divided into three categories for bowel function recovery:≥17 indicated normal bowel function, 12-16 indicated mild bowel dysfunction, and＜12 indicated severe bowel dysfunction.

Continuous variables were presented by mean and standard deviation. Categorical variables were presented as frequencies and percentages.

The medical records of 26 patients were reviewed and listed in table one. Seven were male, and 19 were female, with a mean age of 19.38±13.80 months (ranging from 2 to 47 months).

The anorectal malformation included 17 rectoperineal fistulae without an anus, four rectovestibular fistulae without an anus, two anal stenoses, one anal atresia, one rectourinary fistula without an anus, and one cloaca. X-ray of the sacrum, barium enema, ECG, pelvic ultrasound, and MRI of the pelvis and spine were given as regular preoperative examinations to exclude accompanied anomalies. Megacolon was found in seven patients.

The PS was taken in 23 cases. The SPS was taken in eight cases, and LPS was taken in another 15 patients. SPS group included three rectoperineal fistulae without an anus, one anal stenosis, one retraction of the rectum after an anoplasty for rectovestibular fistula without an anus, one anal atresia, one rectourinary fistula without an anus, and one cloaca. In the LPS group, there were 13 perineal fistulae without an anus，one displacement of the rectum and one retraction of the rectum respectively after an anoplasty for rectovestibular fistula without an anus. TA was used in one anal atresia with megacolon and presacral mass. AS was used in one rectovestibular fistula with a presacral mass. A lamina approach was chosen for one patient with an infective fistula secondary to a presacral mass in the spinal canal. A lamina approach should be considered when there was a request for detethering the spinal cord despite the route for anoplasty.

Eight of the patients had an enterostomy before they came to us. Eight underwent ileostomy in our department. And the other ten patients had no ostomy. The stoma was closed three months after the radical surgery.

The mean follow-up time was 39.48±26.84m, ranging from 6m to 145m. The bowel function score(BFS) was noted regarding the ability to hold back defecation, feeling/ reporting the urge to defecate, and the frequencies of defecation, soiling, fecal accidents, constipation, and social problems. The Rintala score was16.74±2.93, ranging from 12 to 20. 14 patients(14/26, 53.8%) had normal bowel function (BFS ≧ 17), and no patient had severe bowel dysfunction (BFS＜12). Four patients needed oral oxidative or enema to defecate for constipation.

Patients with Currarino syndrome can present with a spectrum of anomalies, including a congenital sacral anomaly associated with a presacral mass and an anorectal malformation, usually anorectal stenosis[10]. In this study, as to ARM, rectoperineal fistula without anus prevailed(17/26, 65.4%), and other anomalies included anal stenosis (2/26, 7.7%), imperforate anus with or without rectourinary fistula(2/26, 7.7%), rectovestibular fistula without anus(4/26, 15.4%), and cloaca(1/26, 3.8%).

The expressivity of this triad is variable, and the clinical manifestation is a complex spectrum of it. Constipation is the most commonly reported symptom. In our 26 cases, 16 patients in this study came for intractable constipation, with eight of them having already undergone an anoplasty elsewhere. Besides, the other complaints were untreated presacral mass, retraction of the rectum after anoplasty, and infection of the presacral mass or infective presacral fistula. So, the complexity of the clinical manifestation was a big challenge to its management.

Management of Currarino syndrome generally consists of treatment of anorectal malformations and presacral mass. The principle of surgical procedure was concerned with correcting the ARM and excising the presacral cyst[7]. As to ARM, the SPS was used to repair a high-type ARM (rectourethral ﬁstula), while the LPS was the technique of choice for the more common low types (rectoperineal/rectovestibular ﬁstula/anal stenosis)[11,12]. Besides, an AS approach and TA approach were also reported^{[3, 5]}. The AS was used for the rectovestibular fistula, and the TA approach was used for anal stenosis. Twenty-three patients used the PS in our study, including eight SPS and 15 LPS. SPS is the classic route to remove the presacral mass and complete the anoplasty with adequate exposure. While LPS just needs to split parts of the external muscle complex and ani levator, which hurts a little to the neurovascular structure around the anus. In our study, all patients with a high-type ARM chose the SPS. And patients with low-type ARM commonly chose the LPS. In five patients who presented with a low-type ARM, we chose an SPS incision when the diameter of the presacral mass was large than 5cm or the position of the normal rectum was high.

When a presacral mass is to be excised, it can usually be done in the same sitting as repairing the ARM. It may be approached surgically through the PS attained by an incision from the sacrum to the anus[13]. Care should be taken during the dissection of the posterior attachments of the mass, when an anterior projection of the thecal sac may be encountered. When anterior sacral meningoceles occur, the traditional practice is to repair the meningocele before correcting the ARM. Nowadays, many pediatric surgeons excise the sac in the same session when using the PS. There were seven presacral teratomas accompanied with anterior meningocele in this group, and five of them were excised using the PS. For the other two, one was resected using the TA, but there was a CSF leakage after the operation, which may be due to the inadequate exposure.

A tethered spinal cord may be associated with Currarino syndrome, but there is no indication for prophylactic spinal cord detethering. If there is an indication to operate on the tethered spinal cord, cord detethering should be considered in the laminar approach. In our series, two cases underwent detethering of the spinal cord, one for a fistula between the presacral mass and spinal canal, the other for parents’ request.

For a rectoperineal fistula, SPS or LPS could be used to reach a complete cutback of the posterior wall of the rectum and remove the presacral mass. For a rectovestibular fistula, the AS or LPS could be chosen. For anorectal stenosis, TA, SPS, or LPS could be used to excise the stenosed bowel segment and the presacral mass.

Seven patients in this group were complicated by megacolon, and five of them used the LPS incision. One of the other two patients used the TA approach, and the other one used AS approach. TA is not so good for the exposure of the presacral mass, so for a larger one(＞5cm) or one connecting with the spinal canal, the LPS or SPS may be the favorite choice. The other patient with a rectovestibular fistula used AS approach, and when the presacral mass is more significant than 5cm, a backward extension of the incision is needed.

Sixteen cases in this group had a stoma during the treatment. Six cases underwent colostomy after birth for imperforate anus. One patient received an ileostomy in a local hospital for intractable constipation. Another case adopted colostomy due to CNS infection after removing the presacral mass. Eight cases received ileostomy in our department after removing the presacral mass and anoplasty to prevent CNS infection and ensure incision healing. The other ten cases didn’t get a stoma. Palliative surgery is initially performed in an infant born with an ARM to divert the colon and allow the patient to mature before definitive surgery. The initial palliative surgery of an imperforate anus includes a colostomy to enable enteric contents to evacuate, while an additional stoma is created for the distal colon[14]. We prefer to choose the terminal ileum for the stoma because it is easier to operate in and will not influence the pelvic cavity. An ileostomy is suggested for cases with a severe scar in the anus or connection with the neural canal.

In the follow-up, particular attention should be paid to bowel function, and, if present, constipation should be treated[15]. Four patients in this group suffered from constipation and needed oral oxidative or enema to defecate. And follow-up for most patients has shown favorable postoperative bowel functional outcomes.

There were 17 cases in this study that came to us with unfavorable results of ARM operation and undiagnosed presacral mass in other institutions. Better knowledge of the different clinical signs and symptoms of Currarino syndrome enhances appropriate imaging and prompt diagnosis of this condition. Then a protocol with reasonable surgical procedures may help avoid undesirable outcomes.

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No competing interests reported.

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published 06 Jan, 2023

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Editorial decision: Major revision
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You are reading this latest preprint version

Selection of Operative Approach in Children with Currarino Syndrome

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Abstract

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